Pulmonary fibrosis predating microscopic polyangiitis by seven years

A 63-year-old man, ex-smoker with renal failure of recent onset was admitted at the respiratory department with massive haemoptysis. Previous X-rays and CT scans showed pulmonary fibrosis of seven-year duration. Subsequently, he developed high fever, large haemoptysis, new infiltrates and respiratory failure despite broad-spectrum antibiotic treatment. Antineutrophilic antibodies of the perinuclear type with specificity against myeloperoxidase were detected and microscopic polyangiitis was diagnosed. Immunosuppressive treatment with methylprednisolone pulses and cyclophosphamide was started with initially favorable response, but later the patient developed a hospital-acquired pneumonia which was treated successfully with meropenem. As pulmonary haemorrhage recurred, he was transferred to intensive care for plasmapheresis which was considered the last treatment option. Unfortunately he died from septic shock.ConclusionAsymptomatic pulmonary fibrosis can predate microscopic polyangiitis by several years and is associated with unfavorable prognosis of the vasculitis. Appreciation of this finding would lead to faster diagnosis and better management of these patients.